title: Treatment of unresectable or metastatic pediatric soft tissue sarcomas with surgery, irradiation, and chemotherapy: a Pediatric Oncology Group study.
abstract: BACKGROUND
  The objectives of this study were to compare vincristine/actinomycin D/cyclophosphamide/adriamycin (VACA) with VACA/plus imidazole carboxamide (DTIC) (VACAD) therapy in regards to complete/partial response and event free survival rates in children and adolescents with metastatic non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) or previously chemotherapy-naive recurrent NRSTS or locally persistent gross residual tumor after surgery and radiation therapy.
  
  
  METHODS
  Between 1986 and March 1994, 75 patients entered this randomized study comparing VACA and VACAD, given at 3 week intervals. Sixty-one patients were considered eligible and received chemotherapy and radiation therapy to the primary tumor and areas of metastases. Thirty-six patients had regional unresected (Group III) disease, and 25 had metastatic disease (Group IV) at time of accession. Thirty-six patients received VACA (11 were not randomized), and 25 received VACAD.
  
  
  RESULTS
  With a median follow-up of greater than 4 years, overall and event-free survival for all eligible patients are 30.6% and 18.4% respectively (S.E: 9.5% and 6.8%). There was insufficient evidence that DTIC offered any advantage to event free survival, but there was evidence for better outcome for patients in Group III disease in comparison to patients with Group IV disease, and for patients with a Grade 1 and 2 histology in comparison to Grade 3 lesions.
  
  
  CONCLUSIONS
  Combination chemotherapy with VACA and VACAD were insufficient to prevent recurrent or progressive disease in children and adolescents with high stage NRSTS. The use of vincristine/ifosfamide/doxorubicin with cytokine support is under study.
authors.affiliation: Department of Hematology-Oncology, St Jude Children's Hospital, Memphis, TN 38105, USA.
authors.forename: C B
authors.initials: CB
authors.lastname: Pratt
authors.forename: H M
authors.initials: HM
authors.lastname: Maurer
authors.forename: P
authors.initials: P
authors.lastname: Gieser
authors.forename: A
authors.initials: A
authors.lastname: Salzberg
authors.forename: B N
authors.initials: BN
authors.lastname: Rao
authors.forename: D
authors.initials: D
authors.lastname: Parham
authors.forename: P R
authors.initials: PR
authors.lastname: Thomas
authors.forename: R B
authors.initials: RB
authors.lastname: Marcus
authors.forename: A
authors.initials: A
authors.lastname: Cantor
authors.forename: T
authors.initials: T
authors.lastname: Pick
authors.forename: D
authors.initials: D
authors.lastname: Green
authors.forename: J
authors.initials: J
authors.lastname: Neff
authors.forename: J J
authors.initials: JJ
authors.lastname: Jenkins
mesh_terms: D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003606:Dacarbazine / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007262:Infusions, Intravenous; D007275:Injections, Intravenous; D008297:Male; D012008:Recurrence; D012509:Sarcoma / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D012983:Soft Tissue Neoplasms / Q000188:drug therapy* / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D003609:Dactinomycin; D014750:Vincristine; D003606:Dacarbazine; D004317:Doxorubicin; D003520:Cyclophosphamide